Growth hormone effects on childrens height with achondroplasia and hypochondroplasia: a systematic review and metanalysis

Introduction: Achondroplasia (Ach) is the most frequent cause of dwarfism. The first therapeutic strategy offered to patients with Ach was. However, GH has played un important role in Ach and Hypochondroplasia (Hch), despite short-term and long-term effects. Purpose: The aim of this systematic review and meta-analysis was to assess the efficacy of GH in the height of patients with Ach and Hch in the short and long term. Methods: 12 studies were included selected from the Pubmed database (3 Randomized Clinical trials (RCTs) and 9 prospective studies) from 1993 to 2014. Comparing high and low doses of GH. The systematic review included 9 prospective studies and the high-dose GH arm of the 3 RCTs. Inclusion criteria was focused on paediatric patients with Ach and Hch treated with GH. Demographic variables were collected including age, gender, dose, height and follow-up. The height variables included height increase and height velocity. Finally, 363 patients with Ach and 41 patients with Hcb were included. A was performed with a follow-up from one to 3 years. Results: In patients with Ach the average height velocity at one, two and three years were 2.65, 1.07 and -0.87 cm/years respectively (p<0.05). The RCTs showed a significant increase in height velocity in patients treated with high dose of GH (MD= 1.38, 95% CI: 0.68-2.07, p=0.0001, I2=0%) . Height at one year increased 0.61 cm. The RCTs did not show significant differences (MD 0.11, 95% CI: 0.17-0.39, p=0.44, I2 = 0%). Finally, patients with Hch increased height velocity 4 cm/year at the first year (p<0.05). Conclusion: GH treatment is beneficial in the shor-term height of children with Ach and Hch. GH effect on different ages and subgroups is unknown, as well as its possible long--term consequences

Resultado a largo plazo de las fusiones largas espinopelvicas en escoliosis: importancia de la edad, la capacidad de marcha y el tipo de fijación ilíaca / Resultado a longo prazo das grandes artrodeses espinopélvicas em escoliose: importância da idade, da capacidade de marcha e do tipo de fixação ilíaca / Long-term result of major spinopelvic arthrodeses in scoliosis: importance of age, walking ability and type of iliac fixation

OBJETIVO: Conocer los factores que influyen en los fracasos instrumentales lumbosacros después de fusiones espinopélvicas largas. MÉTODO: Estudio retrospectivo de pacientes diagnosticados con escoliosis, tratados quirúrgicamente por vía posterior, realizándo se fusión espinopélvica de T2 o T3 a ilíaco, utilizando tornillos pediculares e ilíacos. Se analizaron los fracasos instrumentales y su asociación con diferentes parámetros clínicos y radiológicos. RESULTADOS: Se presentan 44 pacientes con edad promedio de 24 años, con diferentes etiologías. El valor promedio preoperatorio de la curva mayor era de 74,2º, y en la revisión final la corrección promedio fue 67%. El desequilibrio anteroposterior y la inclinación pélvica, la cifosis torácica, la lordosis lumbar y el desequilibrio lateral mejoraron significativamente en la revisión final. Hubo 41% de fracasos de instrumentación, todos a nivel lumbosacro. Se encontró asociación significativa con más fracasos instrumentales en mayores de 17 años y en los que tenían deambulación autónoma. En 24 pacientes, se utilizó un solo tornillo ilíaco bilateralmente y en 20 pacientes, dos o más. Los dos grupos tuvieron una incidencia similar de fracasos. En el grupo de dos o más tornillos solo existieron roturas de barras sin desanclajes, ni lisis. El uso de implantes intersomáticos de L3 a S1 o cerclajes sublaminares con una tercera barra disminuyó la incidencia de fracasos. CONCLUSIONES: En esta serie se presentaron un 41% de fracasos instrumentales, todos localizados a nivel lumbosacro. Los pacientes con capacidad de deambulación autónoma y mayores de 17 años presentaron significativamente más fallas instrumentales. En los que se realizó fusión intersomática L3-S1 o una tercera barra con cerclajes sublaminares, disminuyó la incidencia.

OBJETIVO: Compreender os fatores que influenciam as falhas instrumentais lombossacrais depois de fusões espinopélvicas grandes. MÉTODOS: Estudo retrospectivo de pacientes com diagnóstico de escoliose, tratados cirurgicamente com fusão espinopélvica por via posterior, realizada de T2 ou T3 até o ilíaco, com parafusos pediculares e ilíacos. As falhas instrumentais foram analisadas, além de sua associação com diferentes parâmetros clínicos e radiológicos. RESULTADOS: Apresentamos 44 pacientes com média de idade de 24 anos, com diferentes etiologias. O valor médio da curva pré-operatória maior foi 74,2 graus, e na revisão final foi de 67%. O desequilíbrio anteroposterior e a inclinação pélvica, a cifose torácica, a lordose lombar e o desequilíbrio lateral melhoraram significantemente na revisão final. Houve falhas de instrumentação de 41%, todas na região lombossacral. Verificou-se associação significante com mais falhas instrumentais em pacientes com mais de 17 anos e nos que tinham deambulação independente. Em 24 pacientes, utilizou-se um único parafuso ilíaco bilateral e em 20 pacientes, foram usados dois ou mais. Os dois grupos tiveram incidência de falhas semelhante. No grupo de dois ou mais parafusos ocorreram apenas quebra de hastes, sem perda da ancoragem ou lise. Os implantes intersomáticos de L3 a S1 ou amarrias sublaminares com uma terceira haste diminuíram a incidência de fracassos. CONCLUSÕES: Nesta série ocorreram 41% de falhas instrumentais, todas localizadas na região lombossacral. Os pacientes com capacidade de marcha independente e com mais de 17 anos tiveram significantemente mais falhas instrumentais. Nos pacientes submetidos à fusão intersomática de L3-S1 ou com uma terceira haste com amarrias sublaminares, a incidência foi reduzida.

OBJECTIVE: To determine the factors that influence lumbosacral instrumentation failures following spino-pelvic fusions. METHODS: A retrospective study of patients diagnosed with scoliosis who underwent spinopelvic fusion via posterior, from T2 or T3 proximally to iliac crest, using pedicle and iliac screws. Instrumentation failures were analyzed, and the association of this complication with different parameters was studied. RESULTS: We present 44 patients, with an average age of 24 years, with different etiologies. The mean value of the largest preoperative major curve was 74.2°, and in the final review, it was 67%. The anterior-posterior imbalance, pelvic tilt, thoracic kyphosis, lumbar lordosis and lateral imbalance were significantly improved in the final review. There were instrumentation failures in 41% cases, all at the lumbosacral level. A significant association was found between increased instrumentation failures in patients over 17 years and in patients with independent walking ability. In 24 patients, a bilateral single iliac screw was used and in 20 patients, two or more screws were used. Both groups had a similar incidence of failures. In the group with two or more screws, only rod breakages occurred, without detachment or screw lysis. There was lower instrumentation failure incidence in the patients who underwent L3-S1 interbody fusion or a third rod attached with sublaminar compression hooks. CONCLUSIONS: This series had 41% instrumentation failures, all located at lumbosacral level. There were significant more instrumentation failures in patients with independent walking ability and those aged over 17 years. There was lower instrumentation failure incidence in the patients who underwent L3-S1 interbody fusion or a third rod attached with sublaminar compression hooks.